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Phenylacetate Metabolism
描述
Phenylacetate metabolism is of clinical importance because it facilitates conjugation with glutamine to form phenylacetylglutamine, which can be excreted in the urine. This route provides an alternative pathway for nitrogen excretion in patients with urea cycle defects. This conjugation proceeds in two steps. First, phenylacetate and ATP react with coenzyme A to form phenylacetyl CoA, AMP, and pyrophosphate. In the second step, phenylacetyl CoA and glutamine react to form phenyacetyl glutamine and Coenzyme A.